Evaluation of unresponsiveness to standard high-dose gamma globulin therapy in Kawasaki disease

Objectives: We investigated the incidence of unresponsiveness to intravenous gamma globulin (IVIG) treatment in Kawasaki disease (KD) and evaluated its relation with coronary artery involvement.
Study design: The study included 20 children (13 boys, 7 girls; mean age 4.2±3.4 years; range 9 months to 12 years) with KD. The mean disease duration on admission was 7.3±2.4 days (range 5 to 14 days). Initial treatment consisted of a single dose of IVIG and high-dose of aspirin. Unresponsiveness was defined as the persistence of fever and other symptoms within the first 48 hours of treatment. All the patients were evaluated by two-dimensional echocardiography before and after treatment. The mean follow-up period was 16.5±2.8 months (range 9 to 24 months).
Results: Unresponsiveness was seen in five patients (25%), who received a subsequent dose of IVIG, which improved fever in two patients. The remaining three patients received high-dose methylprednisolone. One patient who showed no response to either IVIG or methylprednisolone was treated with low-dose oral methotrexate. Six patients (30%) had coronary artery involvement (4 dilatations, 2 aneurysms), five patients on admission echocardiography, and one patient on control echocardiography seven days after treatment. Of five unresponsive patients, four (80%) had coronary artery involvement on admission. Patients with coronary involvement underwent coronary angiography after a mean of one year. Five patients had normal coronary arteries, whereas no angiographic regression was observed in one patient who had a giant coronary artery aneurysm on admission and was treated with oral methotrexate.
Conclusion: The incidence of unresponsiveness to treatment was markedly high in KD patients who had coronary artery involvement on admission.

Keywords: Child, coronary disease, echocardiography; immunoglobulins, intravenous/therapeutic use; mucocutaneous lymph node syndrome/drug therapy; treatment failure